{"product_id":"anti-aldh6a1-antibody-picoband-r","title":"Anti-ALDH6A1 Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e: Lyophilized\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e: Human,Mouse,Rat\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e: WB,IHC\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.1-0.25μg\/ml, Human, Mouse, Rat\u003cbr\u003e\nImmunohistochemistry (Paraffin-embedded Section), 2-5μg\/ml, Human\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: ALDH6A1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial (MMSDH) is an enzyme that in humans is encoded by the ALDH6A1 gene. This gene encodes a member of the aldehyde dehydrogenase protein family. The encoded protein is a mitochondrial methylmalonate semialdehyde dehydrogenase that plays a role in the valine and pyrimidine catabolic pathways. This protein catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Alternate splicing results in multiple transcript variants.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: A synthetic peptide corresponding to a sequence at the C-terminus of human ALDH6A1, identical to the related mouse and rat sequences.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Add 0.2ml of distilled water will yield a concentration of 500ug\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 1. Chambliss, K. L., Gray, R. G. F., Rylance, G., Pollitt, R. J., Gibson, K. M. Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency. J. Inherit. Metab. Dis. 23: 497-504, 2000.\n2. Dobrowolski, S. F., Alodaib, A., Karunanidhi, A., Basu, S., Holecko, M., Lichter-Konecki, U., Pappan, K. L., Vockley, J. Clinical, biochemical, mitochondrial, and metabolomic aspects of methylmalonate semialdehyde dehydrogenase deficiency: report of a fifth case. Molec. Genet. Metab. 129: 272-277, 2020.\n3. Gray, R. G. F., Pollitt, R. J., Webley, J. Methylmalonic semialdehyde dehydrogenase deficiency: demonstration of defective valine and beta-alanine metabolism and reduced malonic semialdehyde dehydrogenase activity in cultured fibroblasts. Biochem. Med. Metab. Biol. 38: 121-124, 1987. \u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: Q02252\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHost\u003c\/b\u003e: Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eCross Reactivity\u003c\/b\u003e: No cross-reactivity with other proteins.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e: Rabbit IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePhospho_site\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved Molecular Weight\u003c\/b\u003e: 58 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 4329\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: Methylmalonate-semialdehyde\/malonate-semialdehyde dehydrogenase [acylating], mitochondrial\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: aldehyde dehydrogenase 6 family member A1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: ALDH6A1; MMSADHA; MMSDH\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48787564527716,"sku":"A08868-1","price":94200.0,"currency_code":"JPY","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_a1929566-782c-43d6-acbc-9a5dc8f6ae3f.jpg?v=1784402047","url":"https:\/\/www.genebiosystems.com\/en-jp\/products\/anti-aldh6a1-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}